The urological care of the neurogenic bladder consists of 2 components: medical management with preservation of renal function and quality-oflife issues with achieving dryness and independence of bladder and bowel management. Both components are equally important for patients to live a healthy and fulfilled life. This report explores the diagnosis of the neurogenic bladder; quality-of-life issues that caregivers and patients should expect; the importance of primary care knowledge of the neurogenic bladder and treatment; surgical options; the transition of pediatric patients to adult care; and the importance of caregiver and patient understanding of their disease, treatment options, and responsibilities.
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Before surgery is considered, conservative protocols have to be maximized because two-thirds of patients can become continent by clean intermittent catheterization and medication alone. Fecal continence and bowel management is an integral part of spina bifida treatment.
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Reconstructive lower tract surgery remains the best-studied, most successful, permanent, and long-term satisfactory surgery to deal with urinary and stool incontinence.
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The urological care of patients with spina bifida consists of 2 components: the medical management with preservation of renal function and the quality-of-life issues with achieving dryness and independence of bladder and bowel management. Both components are equally important for patients to live a healthy and fulfilled life.
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Continuous education and care of the families and patients are necessary to motivate them for participation in treatment plans and to avoid frustration and unrealistic expectations.
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Achieving continence is a long road that requires understanding and compliance that is best delivered by a team of specialists.
Neurogenic bladder defects are generally classified according to neurologic defects or functional impairment. Neurologic defects can be described by the Loop theory of Bradley ; in 1974, he differentiated between 4 different loops of neuronal connection. According to his theory, the disruption of each loop causes specific forms of neurogenic bladder dysfunctions. Other investigators based their classifications and observations on urodynamic findings. Also, anatomic and neurologic descriptions help to understand the mechanism of the defect, the functional classification systems have more use for clinical decision making. Especially in children, the most important question remains whether or not the bladder can empty spontaneously while maintaining safe pressures or if drainage has to be provided.
The causes and presentation of a neurogenic bladder in a child is different from adult forms. In most cases, the pediatric neurologic bladder is caused by congenital problems, and many investigators differentiate between a neurologic and a neuropathic bladder. A neurogenic bladder is one from a true neurologic deficit like spina bifida (SB), and a neuropathic bladder is one that acts like a neurogenic bladder but is not caused by an innervation problem. Examples of neuropathic bladders include those caused by a urinary obstruction, like in posterior urethral valves, or lack of bladder tissue, like bladder exstrophy. Acquired forms caused by trauma, infection, or behavioral issues are more comparable with adult clinical pictures. The timing of intervention is important because delay of treatment can cause irreversible damage, especially to the upper tracts. Many congenital defects are obvious, whereas others, like posterior urethral valves, might not be detected until problems occur. Prenatal ultrasounds (US), careful postnatal examination, and the education of primary care providers and parents all help in diagnosing problems early.
Myelodysplasia includes a variety of neural tube defects that differ in their severity depending on site and gravity of the defect. Myelomeningocele (SB) is likely the most common congenital diagnosis for the development of a neurogenic bladder in children. The level of the defect on the spine is strongly associated with survival and the development of cognitive and motor skills, with cervicothoracic levels performing significantly lower in comparison with lumbosacral defects. In contrast to the general motor function, the bladder and bowel function do not have a direct correlation with the level of the lesion. Therefore, even patients with SB with the ability to walk often have poorly functioning bladder and bowels that prevents them from achieving spontaneous continence.
The prevalence of SB in Europe is declining because of prenatal management, but the numbers in the United States remain high, with an estimated 1 per 1000 births in 1993. A newer study of 10 regions in the United States demonstrated a prevalence of SB in children aged 0 to 19 years to be 3.07 per 10,000 in 2002. The total number of cases of the same age group living the United States in 2002 was estimated at 24.860. The aggressive promotion of folic acid supplements during all pregnancies, but especially in families with a history of SB, plays a big role in the decline in numbers of newborns with SB. Today, infant survival rates with SB have increased to 92% in 2001 up from 83% in 1983. As a result of improved surgical treatments and early medical care, children with SB can expect longer lives today than in the past. This encouraging fact also means that primary care providers have to be more knowledgeable about the medical problems because these children not only grow in numbers but also transition into adulthood.
Prenatal diagnosis and fetal surgery
Routine prenatal US examinations allow for earlier diagnosis of affected fetuses with SB conditions. Lately, Chaoui and colleagues reported that they identified 5 cases of SB at 11 to 13 weeks’ gestation by assessing the intracranial translucency and the posterior brain during routine US. A few months earlier, a different group found that at 11 to 13 weeks’ gestation most fetuses with SB have measurable abnormalities in the posterior brain. The early detection of SB fueled the desire for intrauterine repair to allow for regular development and improve the prognosis.
To prospectively evaluate the value of intrauterine surgery, a multicenter, randomized controlled trial was established by the National Institutes of Health and limited to 3 centers in the United States. The endpoints of this Management of Myelomeningocele Study (MOMS) included fetal and infant mortality, the need for a ventriculoperitoneal shunt at 1 year of age, and the evaluation of mental and motor development at 30 months of age.
The study began in 2003 and enrolled 183 women who were pregnant with a fetus with SB. In a randomized fashion, half of the affected individuals underwent fetal surgery, whereas the other half had standard postnatal closure of their defect. The results of the study were published in March 2011 in the New England Journal of Medicine . There were no maternal deaths and the rates of adverse neonatal outcomes were generally similar between the two groups. Two perinatal deaths occurred in each group. The rates for shunt placement were 40% in the prenatal-surgery group and 82% in the postnatal-surgery group. The prenatal surgery group had better motor function compared with the postnatal surgery group, and parent evaluation confirmed these findings. However, there was no significant differences in cognitive scores. Prenatal surgery was associated with an increased risk of preterm delivery and uterine dehiscence at delivery. The data and safety monitoring committee of the study met on December 7, 2010 and recommended termination of the trial because of the efficacy of prenatal surgery. Of great importance is that all 3 centers of the study have a multidisciplinary team of experts and followed a standard protocol to perform fetal surgery. The investigators caution: “The results of this trial should not be generalized to patients who undergo procedures at less experienced centers or who do not meet the eligibility criteria.” No comments were made concerning the effect of the prenatal surgery on the bladder development. Currently, a separate arm of the MOMS trial is looking at bladder function, and results should be available in the next 5 years.
Initial bladder management: expectant versus proactive
The spinal defect is usually closed in the first 24 hours of life and the bladder drained for that period. Concerning the bladder function, 2 general scenarios are observed. One group of patients with SB will have an overactive sphincter and develop high detrusor leak point pressures greater than 40 cm H 2 O and are, therefore, at risk for upper tract damage. The other group will have little sphincter resistance with low detrusor leak point pressures, resulting in free urine flow into the diaper with little risk to the upper tract.
Depending on the policy of the treating institution, 2 different management options are followed. The expectant approach has a more observational and conservative stance, whereas the proactive approach aims to actively influence bladder development. Both options are considered safe for the development of renal function but differ in their perceived benefit for bladder development and later continence. Both approaches require a detailed physical examination, evaluating the presence of a palpable bladder or kidney. Also, a bladder and renal US is obtained to evaluate bladder filling and hydronephrosis. In the case of a negative physical examination and the absence of hydronephrosis, the expectant approach does not initiate clean intermittent catheterization (CIC), video urodynamic studies, or anticholinergic medication. Regular follow-up with examination, creatinine value, and US are initiated, and the parents educated toward the occurrence of urinary tract infections (UTI) and decreased urine output. If hydronephrosis is detected or clinical problems such as infection occur, then urodynamic studies are done to determine if initiation of CIC and pharmacotherapy is needed. The proponents of this approach argue that acting on symptoms is sufficient to protect renal function and it is not justified to have all parents perform CIC.
The proactive protocol advocates for early urodynamic testing to identify high-risk patients. Patients with increased leak point pressures, detrusor sphincter dyssynergia, or noncompliant bladder muscles are placed on CIC every 3 to 4 hours during the day, and anticholinergic therapy with oxybutynin is initiated. The rationale is to promote detrusor muscle relaxation and development by bladder cycling and ensure bladder drainage to protect the upper tracts and prevent hydronephrosis. However, if the bladder environment is safe on urodynamics, some groups will stop CIC and move patients to the expectant protocol. Other groups prefer to continue CIC to keep the child used to catheterization and to ensure proper development. Proponents defend their approach by the fact that most patients will need to perform CIC later to achieve continence and it is easier if the child remains used to the procedure. Prophylactic antibiotics have not been proven to be useful in preventing infections, but no uniform treatment protocols exist.
Initial bladder management: expectant versus proactive
The spinal defect is usually closed in the first 24 hours of life and the bladder drained for that period. Concerning the bladder function, 2 general scenarios are observed. One group of patients with SB will have an overactive sphincter and develop high detrusor leak point pressures greater than 40 cm H 2 O and are, therefore, at risk for upper tract damage. The other group will have little sphincter resistance with low detrusor leak point pressures, resulting in free urine flow into the diaper with little risk to the upper tract.
Depending on the policy of the treating institution, 2 different management options are followed. The expectant approach has a more observational and conservative stance, whereas the proactive approach aims to actively influence bladder development. Both options are considered safe for the development of renal function but differ in their perceived benefit for bladder development and later continence. Both approaches require a detailed physical examination, evaluating the presence of a palpable bladder or kidney. Also, a bladder and renal US is obtained to evaluate bladder filling and hydronephrosis. In the case of a negative physical examination and the absence of hydronephrosis, the expectant approach does not initiate clean intermittent catheterization (CIC), video urodynamic studies, or anticholinergic medication. Regular follow-up with examination, creatinine value, and US are initiated, and the parents educated toward the occurrence of urinary tract infections (UTI) and decreased urine output. If hydronephrosis is detected or clinical problems such as infection occur, then urodynamic studies are done to determine if initiation of CIC and pharmacotherapy is needed. The proponents of this approach argue that acting on symptoms is sufficient to protect renal function and it is not justified to have all parents perform CIC.
The proactive protocol advocates for early urodynamic testing to identify high-risk patients. Patients with increased leak point pressures, detrusor sphincter dyssynergia, or noncompliant bladder muscles are placed on CIC every 3 to 4 hours during the day, and anticholinergic therapy with oxybutynin is initiated. The rationale is to promote detrusor muscle relaxation and development by bladder cycling and ensure bladder drainage to protect the upper tracts and prevent hydronephrosis. However, if the bladder environment is safe on urodynamics, some groups will stop CIC and move patients to the expectant protocol. Other groups prefer to continue CIC to keep the child used to catheterization and to ensure proper development. Proponents defend their approach by the fact that most patients will need to perform CIC later to achieve continence and it is easier if the child remains used to the procedure. Prophylactic antibiotics have not been proven to be useful in preventing infections, but no uniform treatment protocols exist.
Quality of life
The initial management is centered on the primary goal of preserving renal function. As the child gets older, life quality and especially continence becomes more important. It is important for the caregiver to make the family understand that most interventions to achieve continence mean a higher level of involvement for everybody. Medical treatment has to be intensified and the child has to be much more involved. Surgical procedures will turn a previously incontinent bladder into a high-volume reservoir that will need to be catheterized. Basically, the family has to understand that surgeries can turn a current, safe incontinent bladder into a continent but very unsafe reservoir if not emptied regularly. Therefore, before initiating changes and continence procedures, patients have to have a high desire to become dry and are willing to accept a new regiment of protocols. Discussions should include the difference between complete and social continence. In the authors’ practice, social continence is defined as being continent for several hours without leaking and being continent from stool. Minimal leakage during the day or night is accepted, whereas complete continence is defined as dry for 3 to 4 hours, including nighttime dryness.
Most families have heard about available medications and procedures. The authors inform patients and families in a detailed discussion what tests, surgeries, hospital stays, recovery, postoperative teaching, and changes in their daily protocol will occur. These consultations can take hours and often require several visits until the family is ready for change. This time is well invested for both caretakers and patients to avoid misunderstandings, frustrations, and to ensure the best possible outcome. Exposing families to social groups like the Spina Bifida Association is equally important to allow discussion and the exchange of information with peers. Often local organizations exist, such as the Greater Oklahoma Disabled Sports Association ( Godsa.org ), a nonprofit organization that provides sports activities, such as wheelchair basketball. The authors’ practice actively supports and promotes these groups and enrolls families into their programs.
Once the decision is made to initiate changes in the management protocol to achieve dryness, a new baseline is established, including video urodynamic testing, US for the urinary tract, and a kidneys, ureter and bladder x-ray for the bowel. CIC, anticholinergics, and bowel management are maximized and their results reevaluated. It is important to include the bowel in the continence program because constipation will adversely affect the bladder and also address the pressing problem of encopresis. Most patients and families will place the importance of continence from stool before urinary dryness. Urine leakage can usually be contained within a diaper and changed at an opportune time. Unlike urine, stool will smell immediately, causing anxiety, and might prevent families from leaving their home environment.
The addition of specialized pediatric nurse practitioners who work with the families extensively is also helpful. This work includes not only explaining the medications and techniques but also providing individualized CIC sessions that address the specific dexterity needs of the child. Catheter type and size is tailored to the specific needs of patients, and bowel management is maximized with oral and rectal options. Regular follow-up in the clinic and on the telephone is provided. This individualized program is time intensive for families and providers, making them understand what it takes to become dry. Investing time and manpower at this stage will deal with anxiety and frustrations early. This investment will not only save time and money later but will also ensure better health and superior postoperative outcomes if surgery does become necessary. This conservative management will be successful in a large number of patients to achieve complete or at least social continence. However, next to continence, independence from caregivers will play a big role as patients become older. Self-catheterization via the urethra can be difficult depending on body shape and genital anatomy. Applying enemas to one self-effectively is almost impossible even for the most mobile patients. The discussion about available surgeries is initiated at this time.
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